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Wednesday, January 11, 2012

Nephrotic syndrome is a collection of symptoms which
occur because the tiny blood vessels (the glomeruli) in the kidney become
leaky. This allows protein (normally never passed out in the urine) to leave
the body in large amounts.

Description

The glomeruli (a single one is called a glomerulus)
are tiny tufts of capillaries (the smallest type of blood vessels). Glomeruli
are located in the kidneys, where they allow a certain amount of water and waste
products to leave the blood, ultimately to be passed out of the body in the
form of urine. Normally, proteins are unable to pass through the glomerular
filter. Nephrotic syndrome, however, occurs when this filter becomes defective,
allowing large quantities of protein to leave the blood circulation, and pass
out or the body in the urine.

Patients with nephrotic syndrome are from all age
groups, although in children there is an increased risk of the disorder between
the ages of 18 months and four years. In children, boys are more frequently
affected; in adults, the ratio of men to women is closer to equal.

Causes
and symptoms

Nephrotic syndrome can be caused by a number of
different diseases. The common mechanism which seems to cause damage involves
the immune system. For some reason, the immune system seems to become directed
against the person's own kidney. The glomeruli become increasingly leaky as
various substances from the immune system are deposited within the kidney.

A number of different kidney disorders are
associated with nephrotic syndrome, including:

·minimal change disease or MCD
(responsible for about 80% of nephrotic syndrome in children, and about 20% in
adults) MCD is a disorder of the glomeruli

The first symptom of nephrotic syndrome is often
foamy urine. As the syndrome progresses, swelling (edema) is noticed in the
eyelids, hands, feet, knees, scrotum, and abdomen. The patient feels
increasingly weak and fatigued. Appetite is greatly decreased. Over time, the
loss of protein causes the muscles to become weak and small (called muscle
wasting). The patient may note abdominal pain and difficulty breathing. Because
the kidneys are involved in blood pressure regulation, abnormally low or
abnormally high blood pressure may develop.

Over time, the protein loss occurring in nephrotic
syndrome will result in a generally malnourished state. Hair and nails become
brittle, and growth is stunted. Bone becomes weak, and the body begins to lose
other important nutrients (sugar, potassium, calcium). Infection is a serious
and frequent complication, as are disorders of blood clotting. Acute kidney
failure may develop.

Diagnosis

Diagnosis is based first on the laboratory
examination of the urine and the blood. While the urine will reveal significant
quantities of protein, the blood will reveal abnormally low amounts of
circulating proteins. Blood tests will also reveal a high level of cholesterol.
In order to diagnose one of the kidney disorders which cause nephrotic
syndrome, a small sample of the kidney (biopsy) will need to be removed for
examination. This biopsy can be done with a long, very thin needle which is
inserted through the skin under the ribs.

Treatment

Treatment depends on the underlying disorder which
has caused nephrotic syndrome. Medications which dampen down the immune system
are a mainstay of treatment. The first choice is usually a steroid drug (such
as prednisone). Some conditions may require even more potent medications, such
as cyclophosphamide or cyclosporine. Treating the underlying conditions
(lymphoma, cancers, heroine use, infections) which have led to nephrotic
syndrome will often improve the symptoms of nephrotic syndrome as well. Some
patients will require the use of specific medications to control high blood
pressure. Occasionally, the quantity of fluid a patient is allowed to drink is
restricted. Some patients benefit from the use of diuretics (which allow the kidney to produce more
urine) to decrease swelling.

Key
terms

Glomeruli — Tiny tufts of
capillaries which carry blood within the kidneys. The blood is filtered by the
glomeruli. The blood then continues through the circulatory system, but a certain
amount of fluid and specific waste products are filtered out of the blood, to
be removed from the body in the form of urine.

Immune system — The complex system within the body
which serves to fight off harmful invaders, such as bacteria, viruses, fungi.

Kidney failure
— The inability of the kidney to excrete toxic substances from the body.

Prognosis

Prognosis depends on the underlying disorder.
Minimal change disease has the best prognosis of all the kidney disorders, with
90% of all patients responding to treatment. Other types of kidney diseases
have less favorable outcomes, with high rates of progression to kidney failure.
When nephrotic syndrome is caused by another, treatable disorder (infection,
allergic or drug reaction), the prognosis is very good.